Zhao Dan Yongdong, McCann Liza, Hahn Gabriele, Hedrich Christian M
Pediatric Rheumatology, Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, WA, 98105, USA.
Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK.
J Transl Autoimmun. 2021 Mar 20;4:100095. doi: 10.1016/j.jtauto.2021.100095. eCollection 2021.
Chronic nonbacterial osteomyelitis (CNO) is an inflammatory bone disorder that most frequently affects children and adolescents. Chronic recurrent multifocal osteomyelitis (CRMO) is a severe form of CNO, usually characterized by symmetrical inflammatory bone lesions and its waxing and waning character. Sometimes severe and chronic pain can significantly affect the quality of life and psychosocial development of individuals affected. In the absence of prospectively tested and widely accepted diagnostic criteria or disease biomarkers, CNO remains a diagnosis of exclusion, and infections, malignancy and other differentials require consideration (1). The pathophysiology of CNO is not fully understood, but imbalanced cytokine expression and increased inflammasome activation in monocytes from CNO patients contribute to a pro-inflammatory phenotype that contributes to bone inflammation (2). Currently, no medications are licensed for the use in CNO. Most patients show at least some response to nonsteroidal anti-inflammatory drugs, others require more aggressive treatment that can include corticosteroids, cytokine-blocking agents and/or bisphosphonates (3). While under the care of an experienced team and sufficient treatment, the prognosis is good, but some patients will develop sequalae which can include vertebral compression fractures (1).
慢性非细菌性骨髓炎(CNO)是一种炎症性骨病,最常影响儿童和青少年。慢性复发性多灶性骨髓炎(CRMO)是CNO的一种严重形式,通常表现为对称性炎症性骨病变及其病情的起伏变化。有时,严重的慢性疼痛会显著影响患者的生活质量和心理社会发展。由于缺乏经过前瞻性验证且被广泛接受的诊断标准或疾病生物标志物,CNO仍然是一种排除性诊断,需要考虑感染、恶性肿瘤及其他鉴别诊断(1)。CNO的病理生理学尚未完全明确,但CNO患者单核细胞中细胞因子表达失衡和炎性小体激活增加会导致促炎表型,进而引发骨炎症(2)。目前,尚无药物被批准用于治疗CNO。大多数患者对非甾体类抗炎药至少有一定反应,其他患者则需要更积极的治疗,可能包括使用皮质类固醇、细胞因子阻断剂和/或双膦酸盐(3)。在经验丰富的团队护理和充分治疗下,预后良好,但部分患者会出现后遗症,包括椎体压缩性骨折(1)。